Macrophage activation syndrome as initial presentation of systemic lupus erythematosus.
Macrophage activation syndrome (MAS) is known to be a severe and potentially life-threatening complication of rheumatic disorder, especially systemic juvenille rheumatoid arthritis. It is very rare for MAS to be an initial presentation of systemic lupus erythematosus (SLE). Here, we report a 14-year-old girl in whom MAS developed as an initial presentation of SLE. With early diagnosis and administration of cyclosporine A, she had a fair outcome. Further testing showed positive anti-dsDNA about 8 months later.
Yeap
Say-Tin
ST
Department of Pediatrics, Chang Gung Memorial Hospital, Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan.
Sheen
Jiunn-Ming
JM
Kuo
Ho-Chang
HC
Hwang
Kao-Pin
KP
Yang
Kuender D
KD
Yu
Hong-Ren
HR
D000293:Adolescent; D000974:Antibodies, Antinuclear / Q000097:blood; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007154:Immune System Diseases / Q000209:etiology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology; D008262:Macrophage Activation; D012216:Rheumatic Diseases / Q000150:complications*; D013577:Syndrome
D000974:Antibodies, Antinuclear; D016572:Cyclosporine